Paraganglioma Part 5

Last November Sarah and I went to the Huntsman Cancer Institute to have our blood drawn for genetic testing. She for the SDHB gene (something I learned about on two different Facebook groups associated with this disease) that causes paraganglioma and me for a familial melanoma gene. I tested negative for the melanoma gene. In January while crossing the Pacific Ocean (above) with Semester at Sea we received word that Sarah tested positive for SDHB. Given Sarah's positive test, in May we went to Huntsman for more blood drawing. We are now petitioning and waiting for our insurance company (DMBA) to approve running the genetic tests for Joel, Will and Marie (who is the more likely parent to have the gene given that her brother has had a paraganglioma). Once we know if they have the gene or not we will then know what signs of tumors we should look for.

Since Sarah has already had three tumors removed and then tested positive for the gene, one of our first tasks after returning from Semester at Sea was for Sarah to have a MRI. That scan of her body turned up nothing, but blood work at the time indicated that her catecholamine (which include norepinephrine that causes a rise in blood pressure) numbers were up a little. That was enough to warrant a follow up PET scan. Last Friday Marie took Sarah up to Huntsman for her PET scan. We were told that they would call us with the results on Monday. When it didn't come we started to worry.

The call came on Tuesday afternoon (August 4). I was on my way home from a meeting at BYU (another sad story for another day) so Marie got the news. The scan showed no regular tumors, but it did show activity in bone areas which was a pretty good sign that Sarah had paragangliomas growing in her bones and that she most likely had Metastatic Paraganglioma (a unique and rare kind of cancer). The name alone does not sound nice. The call came from our genetic counselor (who knew we were anxious) so she could not give us many details. She told us that they had already scheduled an appointment with a pediatric oncologist (Dr. Jennifer Wright) for Sarah the next morning at 8:30. When appointments are that quick in coming you know that there is probably cause for concern. It was a worrisome evening and night.

At the appointment we were able to see the scan image. The berry flavored radioactive material that Sarah drank on Friday migrates to sugar consuming areas in the body like the brain, the heart and the liver and light them up. It also migrates to paragangliomas. Last time around the PET image clearly showed two of the three tumors. This time, what was shown were bright spots on her upper breast bone, two on her spine and two in her pelvis area. Given Sarah's history, the doctor told us that there was no dilemma about what was wrong. A bone biopsy could verify, but was not necessary. The metastatic part of the diagnosis indicates that these growths in the bone are not in the usual place (the adrenal system) for paraganglioma.

The next step is to start taking an expensive drug used mainly for advanced osteoporosis which will strengthen her bones. If the tumors grow then eventually they can fracture the bones. Then we will meet with her pediatric nephrologist (kidney specialist) to get going on medication to help keep Sarah's blood pressure from too many ups and downs. Then in 6-8 weeks Sarah will have a follow up PET scan. If that scan shows no growth in the five tumors and no new tumors and there are no other symptoms like bone pain then we will wait and see and count our blessings. If the tumor size has grown then several different options will be considered including oral chemo pills, traditional chemotherapy (hair loss and nausea), spot radiation and other IV medications that are designed to kill things that are growing. The doctor described treatment as being like the whack-a-mole game. They will see what pops up and then start to whack at it with whatever seems to be the best weapon. There is no real cure for the tumors. The best hope is that they just sit quietly and do nothing.

One encouraging comment by the doctor is that given the fast pace of cancer research, there will certainly be other treatments over the next few years which could offer more hope. When I asked about whether or not Sarah should plan on trying our for the musical this fall and skiing this winter, she said by all means. When asked about life expectancy, she said it was too early to tell. The only thing she could verify was that it will have an impact. The other encouraging thing about all of this is that without the genetic testing and the scan, we would have had no way of knowing about the tumors. Sarah had noticed a few heart palpitations and had a sense that something was off, but there were no clear signs. It we had to wait until high blood pressure or bone pain forced us to see a doctor, then who knows how far along the cancer would have been at that point and it may have taken lots of doctors and lots of time to even try to figure out what was causing the bone pain. One of the challenges of paraganglioma and pheochromocytoma is that most doctors don't readily associate the symptoms (high blood pressure, bone pain, etc) with these diseases, they always look for the obvious first which means many false starts and wrong paths.

Sarah is full of faith, good humor and quite matter-of-fact that this is something she has to deal with and so she will chose to do it without a lot of angst and fear. In many ways she reminds me of how her grandpa Emmett faced all of his medical challenges. Here is what Marie wrote in her e-mail to the Tueller clan: "In the meantime, we will wait and pray that 1) the tumors are very slow growing and 2) no new ones develop."

For new-comers to this saga, here are links to earlier posts:

 http://beitemmett.blogspot.com/2013/04/paraganglioma.html
 http://beitemmett.blogspot.com/2013/04/gilgal-gardens-and-update-on-sarah.html
 http://beitemmett.blogspot.com/2013/07/paraganglioma-part-2.html
http://beitemmett.blogspot.com/2013/07/paraganglioma-part-4.html
https://beitemmett.blogspot.com/2015/09/paraganglioma-part-6-brothers.html
https://beitemmett.blogspot.com/2015/09/paraganglioma-part-7-its-huge.html

Pacific

Atlantic

Ode to Grandma and Grandpa's House, by Sarah

Tomorrow marks the final clean out of 395 E 600 N in Logan, which for over 50 years has been the home of John and Norda Emmett. Over the past few months the Emmett clan has slowly cleaned out the large house. Mom often lamented that she was leaving lots of drawers and closets to clean out--she did. One day in May the six kids took turns dividing up the treasures. It was a happy, non-contentious event. Once the main items were selected, the rest of the household contents were happily claimed by family members on Memorial Day or when they came by to clean out another closet or drawer. No need for an estate sale or for excessive trips to the DI (Deseret Industries) or the dump. Most everything found a home.

Each trip home from Logan brought more and more boxes of books, paintings, vases, tupperware, quilts, photos..... Last Saturday we brought the big stuff: dinning room table, stuffed chairs, bookcases, quilting frames...... This Wednesday our last two items will be delivered by piano movers: an old pump organ and a player piano. It has become quite a project to try and figure out where to put things, how to rearrange, what to replace and what to get rid off.

Late last Thursday night as I anticipated a two day trip to Logan for the final clean out, a tearful (and tired) Sarah presenting me with this wonderful poem she had just written that so encapsulates the feelings of all of us as we say good by to a place and its peoples that have been such a source of joy and comfort over the years. The things we gathered are mere symbols of the feelings of love, acceptance and support we always felt and will always treasure.


Grandma and Grandpa's House, by Sarah Emmett

What to take, What to take,
From my dead Grandma's things?
A piano from the parlor?
Her necklaces and rings?
Quilts from the basement beds
So squeaky in the night--
The beds that kept our secrets,
And forgot our cousin fights?
Toys from the playroom,
Books from the shelf,
Every tatted snowflake,
And Christmas tree, and elf?
Well worn skis and bread pans,
Garden hoes and rakes,
Furniture and dishes?
What to take, oh what to take?

Can't I take the kisses
My grandpa roughly gave?
Or the sound of grandma's stories
When we visited his grave?
Can't I take the special smell
Of soda, grass, and cars,
That always filled the big garage
And said "this place is ours"?
The sound of uncles' chatter
And friendly shouts Hello?
The atmosphere of coziness,
The winter's chilly glow?
The taste of turkey, salad, jam--
And ice cream in the heat,
Grandma's soft and sweatered hugs,
The holy Grandpa seat?
The excitement of the journey,
The final kiss goodbye?
We always spoke of "next time"

But instead they had to die.

And when I see them both again,
I'll still miss that aging hall,
Where babies cooed,
And children romped,
And John and Norda loved us all.

 "favorite uncle" Chad reading to nieces in grandpa's chair.

 Joel as Joseph

 Will as a shepherd

 John William Emmett and William Blaine Emmett in the "holy grandpa seat."

80th birthday

Sarah and cousins.

addendum: a few more photos from the last day.

Watching this last pool game is when I really teared up. I remember when I was young like this and a whole was cut through our garage wall so the table could bypass the dog leg in the stairs and be brought down into the basement.

 The last supper in the home (by Jake Emmett)


 Followed by one last evening of swapping funny and touching stories.

Family Photos

Photos from June 2015 in Hobble Creek Canyon, 15 minutes from out home. Two years ago, Joel, Sarah and I came here for a church youth activity where we used PVC blow guns to fire marshmallows at each other. Others come here for paint ball wars. We took the photos the night before Joel got his braces on. Thoughts on which ones we should print and hang in our home?

Photos by:  pinklemonadephoto@gmail.com
http://www.pink-lemonade-photo.com/ 

Silver Lake

For our celebration of Pioneer Day (July 24th) we finally decided (the kids were indifferent on most suggested activities) to hike to Silver Lake up American Fork Canyon. Our early morning departure was thwarted by Joel and Will sleeping to 11:00 AM so we post-poned the hike until the 25th. We started our hike at an empty Silver Lake Flat Reservoir. Mt. Timpanogos looms in the distance.

Our destination was Silver Lake in the Lone Peak Wilderness Area (No ATVs allowed). We had read in hiking guides that it was about a 4 mile round trip, but this map showed it as 3.7 miles one way. That distance did not please Will. When we asked one of the returning hikers how long of a hike it was and they said 3 hours, Will was even less happy. But we forged on amid much complaining.

It was at this very point that Will told me he hated me and he hated this hike.

But hey, the wild flowers were pretty.

Luckily we soon inquired of two nice female forest rangers coming down the trail and they told us the 3.7 miles was wrong, that we were about 3/8ths there and it was not up and over the distant peaks, but just beyond the tall pines on the hill crest. Then two other hikers told us that they had seen a moose and calf and they had gone swimming in the cold water. Bingo. Will was now on board. No more complaining. It was a night and day change of attitude. Happy day for all of us.

It was worth the hike.

The marshy shore in the distance is where the moose were. By the time we got there they had moved up into the larger brush.

Cold water and sharp rocks. Next time I'll bring Tevas to wear and quick drying clothing to wear swimming.

Will got his hard cast on last Thursday so his buckle fracture of his left radius will heal. It will be on for two weeks. He can get the cast wet, but only in pool or bath water.

Joel braved jumping into the cold water twice.

Lots of granite, sub-alpine firs and yellow wild flowers.

These falls come out of the lake.

 Chad + Marie did not leave their mark.

 Today in Primary, Will had to give a talk. With only a little help from his parents, this is what he came up with:

Yesterday our family went on a hike up American Fork Canyon to Silver Lake.  I did not want to go.   I didn’t want to get up early and I was acting like a teenager.  I complained a lot.  I thought it would be boring.  We saw one hiker who told us that it would take us three hours.  That make me not want to go even more.  At the start I was trudging along, moaning and groaning.  But then some forest rangers told us that it really wasn’t that long.  We also met some hikers who said they had seen a moose and its calf and that they had gone swimming in the cold water. My mood changed completely.  I wanted to go and see. I started enjoying the hike and the lake really “rocked.”

I am glad that I followed the advice of these people because it led me to a beautiful and fun place. 

The day after Jesus was baptized he told Philip to “Follow Me. “ Philip then told Nathanael that they had found the Messiah, who was named Jesus from Nazareth. Nathanael then asked.  “Can any good thing come out of Nazareth?” Philip then said to him, “Come and see.”

If we come and see what Jesus taught and follow his example we will find happiness in our lives.

For example we can be nice to people at school, even though they don’t like to do the same things we like to do.

We can do better at telling the truth even when we don’t want to.

We can be happy when we are doing our chores.

We can be more reverent in Primary. (Based on how noisy senior Primary was at church last week, Dad added this part, but Will crossed it out and refused to say it in the talk)

I know that if we come and follow Jesus then he will bless us and we will be happy.

I hope we can all follow Jesus.

In the name of Jesus Christ, Amen.

Our drive up the canyon was the inaugural off-road drive of our new car--a 2015 Subaru outback which replaces our 1997 Outback that now will be Sarah's mode of transportation to early morning seminary and late night musical practices. After two tows down the Cottonwood canyons in the past few years, I figured we needed a more reliable ski car for our quest this year to ski all 15 ski areas in Utah.

In mid-July both Joel and Will participated in two day three-on-three soccer tournaments.

Will's team got first place and Joel's team fourth place.

Throughout July we have also enjoyed many gatherings with the coming-and-going-Tuellers. Will broke his arm while tubing behind his uncle Lant's boat at Deer Creek Reservoir. The tube made a big bounce crossing the wake and Will held on so tight that his bone buckled.

Everyone shared a joke or funny story.

Marie's parents with six of their ten children.

Lots of cousins.


Marie's famous scotcheroos, chocolate chip chewies, and rice crispie treats plus Grandma Emmett's recipe for homemade strawberry ice cream.

For our late starting pioneer Day outing we went to the celebration at Provo's North Park. There we visited the Pioneer museum. I liked this painting of pioneer Provo.

It was also cool to notice many pioneer era items that were similar to the pioneer era furniture and other items that my parents had collected over the years. My parents had two antique pump organs in our home and if we can get the one out of the basement next week, it will come to reside in our home.